Cystic fibrosis (CF) results in chronic pulmonary infections, inflammation, pancreatic insufficiency, and multiple gastrointestinal manifestations. Malnutrition and poor growth are hallmarks of CF, and strongly associated with poor outcomes. Through newborn screening, many infants can be diagnosed within a few days of life, which allows for early initiation of nutritional counseling and close clinical follow-up. Obstacles to growth for infants with CF start in utero, as newborns with CF can have a lower birth weight than the general population. Improving infant growth has been linked to improved clinical outcomes and survival. It remains a top priority and challenge for caregivers and healthcare teams. An interdisciplinary approach, including registered dietitian and social work support, is essential to optimize health for infants with CF. Remaining barriers to normalcy include deficits in linear growth, lack of accurate nutrition biomarkers, persistence of inequities related to social determinant of health, particularly in the global CF community.
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http://dx.doi.org/10.1002/ppul.26871 | DOI Listing |
Elife
January 2025
Department of Neurology, Weill Institute for Neuroscience, University of California San Francisco, San Francisco, United States.
Mutations in Sonic Hedgehog (SHH) signaling pathway genes, for example, (SUFU), drive granule neuron precursors (GNP) to form medulloblastomas (MB). However, how different molecular lesions in the Shh pathway drive transformation is frequently unclear, and mutations in the cerebellum seem distinct. In this study, we show that fibroblast growth factor 5 (FGF5) signaling is integral for many infantile MB cases and that expression is uniquely upregulated in infantile MB tumors.
View Article and Find Full Text PDFFront Cardiovasc Med
January 2025
Department of Congenital Heart Disease-Pediatric Cardiology, Deutsches Herzzentrum der Charité (DHZC), Berlin, Germany.
Objective: Interstage home monitoring (IHM) programs are considered standard of care after Norwood palliation and have led to substantial improvements in clinical outcomes. This study aims to evaluate an application-based remote IHM program for infants with shunt- or duct-dependent pulmonary circulation. The primary goals were to discharge infants from the hospital while minimizing mortality, optimizing somatic growth, and enhancing caregivers' confidence in the clinical management at home.
View Article and Find Full Text PDFEndocr Rev
January 2025
Dep. of Clinical Medicine, Aarhus University, Denmark.
Vitamin D deficiency during pregnancy is associated with an increased risk of health issues in the offspring. Accordingly, recent Endocrine Society guidelines strongly support supplementation in pregnancy, also underlining that without consensus on optimal maternal vitamin D levels, routine screening is currently irrelevant. Knowledge of organ-specific effects of vitamin D and its association with maternal vitamin D status may aid to optimize vitamin D supplementation.
View Article and Find Full Text PDFBMC Public Health
January 2025
School of Health and Allied Sciences, Pokhara University, Kaski, Nepal.
Background: Minimum acceptable diet (MAD) intake is crucial for optimizing the growth and development of children aged 6 to 23 months. However, little is known about the factors influencing MAD intake among children of Sudurpaschim. This study, therefore, aims to assess the prevalence of minimum acceptable diet intake and associated factors among children aged 6-23 months in Sudurpaschim Province of Nepal.
View Article and Find Full Text PDFBMC Pregnancy Childbirth
January 2025
Centre for Clinical Epidemiology, Lady Davis Research Institute, Montreal, Québec, Canada.
Background: Lower maternal insulin sensitivity during pregnancy is associated with greater fetal adiposity. Physical activity can improve insulin sensitivity, but it is not known if physical behaviours influence the known association of maternal insulin sensitivity with offspring adiposity. This study aimed to investigate the moderating impact of physical behaviours on this association.
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